Biliary Atresia Flashcards
Biliary = bile, bile ducts or gall bladder
Atresia = absence of lumen or blockage of extrahepatic biliary system
Biliary atresia therefore relates to the absence of a bile duct or a blockage. This is typically a condition of newborns. Which 2 of the following are the most common causes?
1 - trauma during delivery
2 - congenital defect
3 - inflammation of bile duct
4 - increased abdominal pressure
2 - congenital defect
3 - inflammation of bile duct
Viral or toxins are suggested to cause inflammation of bile duct leading to biliary atresia
CFC1 gene has also been linked to this
Biliary atresia therefore relates to the absence of a bile duct or a blockage. This is typically a condition of newborns. Is this more common in males or females?
- females
What is the incidence of biliary atresia?
1 - 1 in every 10,000-15,000 live births
2 - 10 in every 10,000-15,000 live births
3 - 100 in every 10,000-15,000 live births
4 - 1000 in every 10,000-15,000 live births
1 - 1 in every 10,000-15,000 live births
Typically when does biliary atresia present clinically?
1 - 1-2 days from birth
2 - 2-4 weeks from birth
3 - 2-8 weeks from birth
4 - 8-12 weeks from birth
3 - 2-8 weeks from birth
There are 3 types of biliary atresia. Which of the following is a type 1?
3 - atresia of the left and right ducts to the level of the porta hepatis, this occurs in >90% of cases of biliary atresia
2 - proximal ducts are patent, however, the common duct is obliterated
3 - atresia of the cystic duct and cystic structures are found in the porta hepatis
2 - proximal ducts are patent, however, the common duct is obliterated
There are 3 types of biliary atresia. Which of the following is a type 2?
3 - atresia of the left and right ducts to the level of the porta hepatis, this occurs in >90% of cases of biliary atresia
2 - proximal ducts are patent, however, the common duct is obliterated
3 - atresia of the cystic duct and cystic structures are found in the porta hepatis
3 - atresia of the cystic duct and cystic structures are found in the porta hepatis
There are 3 types of biliary atresia. Which of the following is a type 2?
1 - atresia of the left and right ducts to the level of the porta hepatis, this occurs in >90% of cases of biliary atresia
2 - proximal ducts are patent, however, the common duct is obliterated
3 - atresia of the cystic duct and cystic structures are found in the porta hepatis
1 - atresia of the left and right ducts to the level of the porta hepatis
Accounts for 90% of biliary atresia cases
All of the following are common in biliary atresia, but which of the following is the most common by far?
1 - Jaundice
2 - Dark urine
3 - Pale stools
4 - Appetite and growth disturbance
5 - Cardiac murmurs
1 - Jaundice
Conjugated bilirubin spills into the blood causing yellowing
Jaundice is a tell tale sign that a patient may have biliary atresia, however, this can also be non-pathological. Jaundice has to persist for more than what to be considered pathological?
1 - 2h
2 - 2 days
3 - 2 weeks
4 - 2 months
3 - 2 weeks
Which 2 of the following organs can become enlarged in biliary atresia?
1 - spleen
2 - liver
3 - kidneys
4 - appendix
1 - spleen
2 - liver
Hepato and splenomegaly are common
Which 2 of the following are the most effective in diagnosing biliary atresia?
1 - FBC
2 - unconjugated bilirubin
3 - conjugated bilirubin
4 - LFTs
3 - conjugated bilirubin
4 - LFTs
Total bilirubin may be normal, but conjugated will be high
Which of the following may cause biliary atresia?
1 - antiphospholipid syndrome
2 - serum alpha 1-antitrypsin deficiency
3 - elastase deficiency
4 - surfactant deficiency
2 - serum alpha 1-antitrypsin deficiency
Which imaging is often the 1st line for diagnosing biliary atresia?
1 - magnetic resonance cholangiopancreatography (MRCP)
2 - endoscopic retrograde cholangiopancreatography (ERCP) 3 - ultrasound
4 - X-ray
3 - ultrasound
ERCP can be diagnostic and therapeutic
What is the only definitive curative treatment for biliary atresia?
1 - antibiotics and fluids
2 - endoscopic retrograde cholangiopancreatography (ERCP)
3 - surgery
4 - wait and see
3 - surgery
- with antibiotics
Kasai procedure:
- removal of damaged bile ducts
- small intestine attached to liver
- stomach attached to small intestine
Surgery is the only curative therapy for biliary atresia, using the Kasai procedure. Which of the following are complications?
1 - unsuccessful anastomosis formation
2 - progressive liver disease
3 - cirrhosis with eventual hepatocellular carcinoma
4 - all of the above
4 - all of the above
If surgery is good prognosis is good
Liver transplant may be needed in 1st 2 years of life is surgery fails
